Objectives: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized disease. Kidney and retroperitoneum are known to be one of the organs frequently involved. This research aimed to demonstrate the clinical features and outcomes of IgG4-related kidney disease (IgG4-RKD) and IgG4-related retroperitoneal fibrosis (IgG4-RPF). Methods: Patients who satisfied the comprehensive diagnostic criteria on IgG4-RD from January 2009 to July 2020 at three medical institutions were included. They were classified into three groups as RKD, RPF, and Others group. The differences in symptoms, laboratory, histological and radiological findings, treatment, and final outcomes among three groups were evaluated. Results: The number of newly diagnosed patients increased every year. Of total 94 patients, 13 (13.8 %) and 22 (23.4 %) patients were classified into the RKD and RPF group, respectively. The mean age at diagnosis in the RKD (62.2 ± 10.8 years) and the RPF groups (60.1 ± 15.2 years) was older than that in the Others group (51.1 ± 13.3 year) (P = 0.004). Eosinophilia was more common in the RKD group (38.5%) compared to the RPF (9.1%) or the Others groups (8.5%) (P =0.011). Serum IgG levels were no differences among groups. In the RKD group, the histological findings of all renal tissue were tubulointerstitial nephritis. In logistic regressions analysis, hypocomplementemia and renal function at the time of diagnosis were associated with renal involvement (Odds ratio 0.946 and 14.044, respectively). Older age, male and higher serum IgG4 levels were associated with retroperitoneal involvement (Odds ratio 1.054, 6.114 and 1.001, respectively). Glucocorticoids therapy was the most common treatment with no differences among groups. Final response rate was similar among groups. Conclusions: Kidney and retroperitoneum are the major organs of IgG4-RD and they exhibit clinical features that distinguish them from others. Establishment universal consensus for the various subsets of IgG4-RD will be needed in the future.